Autoimmune diseases in myelodysplastic syndrome favors patients survival: A case control study and literature review

Background

We conducted a monocentric retrospective study of patients with myelodysplastic syndromes (MDS) and autoimmune or inflammatory disorders (AIMs) and a literature review. We analyzed the association with subgroups of the WHO 2016 MDS classification and patient's survival in a case control study. Risk factors associated with survival were analyzed by uni- and multivariate analysis.

Results

From all MDS patients 11% presented with AIMs. These were heterogeneous and the most frequent where polyarthritis (25%) and autoimmune cytopenias (17%). No difference for frequency and type of AIMs was observed for the WHO 2016 MDS subgroups (p?=?.3). In the case control study WHO classification, karyotype abnormalities, IPSS-R and IPSS were similar in both groups. The overall survival from MDS diagnosis was better in the group with AIMs 10.3?±?0.6 (IC95% 6.2–12.9) versus 4.8?±?1.1?years (IC95% 4.2–8.7), p?=?.04]. The better survival was restricted to MDS with low or intermediate-1 IPSS 11.1?±?1.5 (IC95% 9.9-NR) versus 8.7?±?1.3?years (IC95% 4.8–10.3), p?=?.006]. The better survival was only observed when AIMs diagnosis was timely associated or appeared after MDS diagnosis (p?=?.04). Factors associated with a better overall survival and survival without AML were steroid dependence respectively HR?=?0.042, p?=?.003, (IC95% 0.005–0.33) and HR?=?0.07, p?=?.002, (IC95% 0.013–0.39)], a diagnosis of AIMs and MDS timely associated respectively HR?=?0.05, p?=?.009, (IC95% 0.006–0.478) and HR?=?0.1, p?=?.008, (IC95% 0.018–0.54)] or a diagnosis of AIMs after MDS respectively HR?=?0.024, p?=?.009, (IC95% 0.001–0.39) and HR?=?0.04, p?=?.008, (IC95% 0.003–0.43)].

Conclusion

Autoimmune and inflammatory diseases associated to MDS are heterogeneous. AIMs diagnosed after or concomitantly to MDS seems associated with a better survival. Prospective studies are necessary to demonstrate that autoimmunity is associated to a better control of the MDS clone.